Highlights
- •Linear scleroderma en coup de sabre presents with sclerotic lesions of the skin.
- •Subcortical white matter lesions are ipsilateral to craniofacial scleroderma.
- •Hemiplegic migraine induced by exercise is a rare neurological manifestation.
- •Some patients require aggressive immunosuppression.
- •There is evidence of central nervous system inflammation and vasculitis.
Abstract
We report the case of a 9-year-old girl with linear scleroderma en coup de sabre (LSCS) who developed progressive white matter involvement, presenting as intractable
hemiplegic migraine-like attacks induced by exercise. After a period of severely aggressive
course, clinical and radiological stabilization was achieved under immunosuppressant
treatment. Intrathecal synthesis of IgG and lymphocytic pleocytosis provided indirect
evidence of a chronic inflammatory process of the central nervous system. We discuss
the possible immunopathogenic mechanisms responsible for the neurocutaneous involvement
in LSCS, favouring the hypothesis of an autoimmune and inflammatory vasculopathy.
The singular occurrence of hemiplegic migraine triggered by exertion add further insight
to the currently unknown pathogenesis of scleroderma disorder. In addition, we highlight
the importance of intensive immunosuppression approaches in selected cases, contrasting
with the classic benign course of LCSC.
Keywords
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References
- Neuroimaging findings in scleroderma en coup de sabre.Neurology. 2004; 62: 1585-1589https://doi.org/10.1212/01.WNL.0000124518.25087.18
- Migraine aura pathophysiology: the role of blood vessels and microembolisation.Lancet Neurol. 2010; 9: 309-317https://doi.org/10.1016/S1474-4422(09)70358-8
- Neurologic manifestations of localized scleroderma: a case report and literature review.Neurology. 2008; 71: 1538-1545https://doi.org/10.1212/01.wnl.0000334474.88923.e3
- Scleroderma “en coup de sabre:” pathological evidence of intracerebral inflammation.J Neurol. Neurosurg. Psychiatry. 2001; 70: 382-385
- Hemiplegic migraine and progressive hemifacial atrophy.Ann. Neurol. 1985; 17: 620
Article info
Publication history
Published online: October 18, 2019
Accepted:
October 18,
2019
Received in revised form:
September 29,
2019
Received:
April 11,
2019
Identification
Copyright
© 2019 Elsevier B.V. All rights reserved.
